Lecture

Motor Neuron Diseases: Pathophysiology & Therapies

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In course

BIO-480: Neuroscience: from molecular mechanisms to disease

The goal of the course is to guide students through the essential aspects of molecular neuroscience and neurodegenerative diseases. The student will gain the ability to dissect the molecular basis of disease in the nervous system in order to begin to under ...

Description

This lecture covers the motor system, motor neuron diseases, and amyotrophic lateral sclerosis. It explains the control of voluntary movements, movement execution, and the role of the basal ganglia in the motor system. The lecture also delves into the organization of the motor system, upper and lower motor neurons, and the descending tracts in the spinal cord. Additionally, it discusses the motor cortex, spinal cord organization across mammalian species, and the neuromuscular system. The presentation concludes with insights into the spinal cord circuit for movement control, motor units, and the neuromuscular junction.

Instructors (4)

Bernard Schneider

Teaching & PhD PhD Students Yesica Gloria, Christian Schellhaas Past EPFL PhD Students Vanessa Laversenne, Sameer Mohammad Khaleel Nazeeruddin, Emma Charlotta Källstig Past EPFL PhD Students as codirector Philippe Guillaume Coune, Emilda Pino, Elisabeth Anne Dirren, Aurélien Lathuilière, Wojciech Janusz Bobela, Lu Zheng, Cylia Cloé Rochat Courses Methods: from disease models to therapy BIOENG-518 This course will describe methods underlying translational approaches from disease modeling an

Hilal Lashuel

2012-2013 Visiting Professor, Standford University. Stanford School of Medicine 2011- Associate Professor of Life Sciences-Brain Mind Institute-EPFL Dir. Laboratory of Chemical Biology of Neurodegeneration 2005-2011 Assistant Professor of Life Sciences-Brain Mind Institute-EPFL Dir. Laboratory of Molecular Neurobiology and Neuroproteomics 2005-2008 Director- EPFL Proteomic Core Facility 2002-2004 Instructor of Neurology- Harvard Medical School and Brigham and Women's Hospital 2001-2002 Sabbatical Fellow- Laboratory for Drug Discovery in Neurodegeneration Harvard Medical School, 2001-2002 Post-doctoral Fellow- Center for Neurologic Diseases Harvard Medical School and Brigham and Women's Hospital Advisor- Prof. Peter T. Lansbury 2000-2001 Research Scientist, The Picower Institute for Medical Research, Great Neck New York 1994-2000 PhD Student; Texas A&M University and the Scripps Research Institute Advisor- Prof. Jeffery W. Kelly 1990-1994 B.S. City University of New York, Brooklyn College Dr. Hilal A. Lashuel received his B.Sc. degree in chemistry from the City University of New York in 1994 and completed his doctoral studies at Texas A&M University and the Scripps Research Institute in 2000. After obtaining his doctoral degree, he became a research fellow at the Picower Institute for Medical Research in Long Island New York. In 2001, he moved to Harvard Medical School and the Brigham and Women's Hospital as a research fellow in the Center for Neurologic Diseases and was later promoted to an instructor in neurology at Harvard Medical School. During his tenure (2001-2004) at Harvard Medical School his work focused on understanding the mechanisms of protein misfolding and fibrillogenesis and the role of these processes in the pathogenesis of Parkinson's and Alzheimer's disease. In 2005 Dr. Lashuel moved Switzerland to join the Brain Mind Institute at the Swiss Federal Institute of Technology Lausanne as a tenure-track assistant professor in neurosciences. Currently, Dr. Lashuel is an associate professor of life sciences and the director of the laboratory of molecular and chemical biology of neurodegeneration. (http://lashuel-lab.epfl.ch/). Research efforts in the Lashuels laboratory focus on understanding the molecular mechanisms of neurodegeneration and developing novel strategies to diagnose and treat neurodegenerative diseases such as Alzheimers and Parkinsons disease. Research in the Lashuel lab is funded by several international funding agencies and foundations, including the Swiss National Science Foundation, European FP7 program (Marie Curie and ERC grants), Human Science Frontiers, Strauss Foundation, Cure the Huntingtons disease foundation and Michael J Fox foundation and is supported by collaborations with pharmaceutical and biotech companies (http://lashuel-lab.epfl.ch/page-50538-en.html), Nestle, Merck-Serono, AC Immune and Johnson and Johnson. Dr. Lashuels research has resulted in the characterization of novel quaternary structure intermediates on the amyloid pathway, identification of potential therapeutic targets, and new hypotheses concerning the mechanisms of pathogenesis in Alzheimers disease, Parkinsons disease and related disorders. Dr. Lashuel scientific contribution to this field includes i) more than100 publications in major peer reviewed journals including Nature journals, Cell, PNAS, JBC, J. Neuroscience JACS, and Angewandtie Chemie; ii) three patents on novel strategies for preventing protein aggregation and treating autoimmune and inflammatory diseases; iii) more than 150 invited lectures since 2002 and more than 5500 citations (7800 citation-Google Scholar) since 1996. Dr. Lashuel has received several pre-doctoral and post-doctoral awards and fellowships and was the recipient of two prestigious awards given to young investigators; Human Science Frontiers young investigator research award and the European Research Council (ERC) starting independent researcher grant and the ERC proof of concept award (2013) These awards provide more than $2.5 Million to Dr. Lashuel to translate some of his ideas and projects into novel strategies for diagnosing and treating neurodegenerative diseases such as Alzheimers and Parkinsons disease. Dr. Lashuel has chaired and co-organized several international conferences and serves as an academic editor for PLoS ONE, an associate editor for frontiers of molecular neuroscience, member of the Editorial advisory board of ChemBioChem and ad hoc reviewer for several international scientific journals and funding agencies.

Official source

Ontological neighbourhood

Biology

Anatomy: Neuroanatomy

Related concepts (31)

Alpha motor neuron

Alpha (α) motor neurons (also called alpha motoneurons), are large, multipolar lower motor neurons of the brainstem and spinal cord. They innervate extrafusal muscle fibers of skeletal muscle and are directly responsible for initiating their contraction. Alpha motor neurons are distinct from gamma motor neurons, which innervate intrafusal muscle fibers of muscle spindles. While their cell bodies are found in the central nervous system (CNS), α motor neurons are also considered part of the somatic nervous system—a branch of the peripheral nervous system (PNS)—because their axons extend into the periphery to innervate skeletal muscles.

Motor system

The motor system is the set of central and peripheral structures in the nervous system that support motor functions, i.e. movement. Peripheral structures may include skeletal muscles and neural connections with muscle tissues. Central structures include cerebral cortex, brainstem, spinal cord, pyramidal system including the upper motor neurons, extrapyramidal system, cerebellum, and the lower motor neurons in the brainstem and the spinal cord. The motor system is a biological system with close ties to the muscular system and the circulatory system.

Reticular formation

The reticular formation is a set of interconnected nuclei that are located throughout the brainstem. It is not anatomically well defined, because it includes neurons located in different parts of the brain. The neurons of the reticular formation make up a complex set of networks in the core of the brainstem that extend from the upper part of the midbrain to the lower part of the medulla oblongata. The reticular formation includes ascending pathways to the cortex in the ascending reticular activating system (ARAS) and descending pathways to the spinal cord via the reticulospinal tracts.

AC motor

An AC motor is an electric motor driven by an alternating current (AC). The AC motor commonly consists of two basic parts, an outside stator having coils supplied with alternating current to produce a rotating magnetic field, and an inside rotor attached to the output shaft producing a second rotating magnetic field. The rotor magnetic field may be produced by permanent magnets, reluctance saliency, or DC or AC electrical windings.

Neuromuscular disease

A neuromuscular disease is any disease affecting the peripheral nervous system (PNS), the neuromuscular junction, or skeletal muscle, all of which are components of the motor unit. Damage to any of these structures can cause muscle atrophy and weakness. Issues with sensation can also occur. Neuromuscular diseases can be acquired or genetic. Mutations of more than 500 genes have shown to be causes of neuromuscular diseases.

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